This chapter talks about a 62-year-old right-handed lady who consulted her family doctor when she noticed that she was unable to use her knitting needle properly with her left hand. The provisional diagnosis was Corticobasal Syndrome (CBS) based on the combination of subtle asymmetric extrapyramidal findings and prominent apraxia. CBS is an akinetic-rigid clinical syndrome characterized by lateralized motor and cognitive signs, such as rigidity, dystonia, spontaneous or stimulus-sensitive myoclonus, apraxia, alien limb phenomenon, cortical sensory loss, and aphasia. This clinical syndrome has been associated with many pathological entities other than CBD, including Progressive Supranuclear Palsy (PSP), Alzheimer's disease, Creutzfeldt-Jakob disease, and many diseases in the spectrum of Frontotemporal Lobar Degeneration (FTLD): Pick's disease, Frontotemporal Dementia (FTD) with ubiquitin-positive inclusions, FTD due to mutations of MAPT and PGRN. Ante-mortem diagnosis can be difficult because proposed criteria lack sensitivity and specificity.